Abernethy malformation type 2: varied presentation, management and outcome

doi:10.1016/j.jpedsurg.2018.08.053

Journal of Pediatric Surgery

Volume 54, Issue 4, April 2019, Pages 760-765

https://doi.org/10.1016/j.jpedsurg.2018.08.053 Get rights and content

Abstract

Purpose

To study the varied presentations and the outcomes in children with Type 2 Abernethy malformation following shunt ligation.

Material and methods

Children with Type 2 Abernethy who had had been operated between 2013 and 2017 were included in the study. The diagnosis had been confirmed on ultrasonography, CECT or angiography. All patients underwent laparotomy. The shunt was identified, clamped and the bowel congestion was noted. The shunt was ligated if the bowel congestion was not significant or had improved. Relevant follow-up investigations were done to document the resolution or amelioration of symptoms and the patency of the shunt.

Results

Five patients were included in the study with a median age of 6 years. Hepatopulmonary syndrome was the presentation in 4 patients while one patient presented with liver tumor. Ultrasonography and CECT were able to diagnose Type 2 malformation in 4 patients whereas in 1 patient the distal portal vein was not seen. The postoperative period was complicated in 3 patients. At the median follow up at 14 months, good intrahepatic portal flow in all patients. All patients demonstrated improvement/ resolution of symptoms.

Conclusion

Abernethy is rare malformation which can have a varied presentation. Additional investigations may be needed to confirm the diagnosis of Type 2 variety. Most patients have gradual improvement of symptoms.

Level of evidence

Level IV/ Treatment study.

Section snippets

Study design

A retrospective study was conducted in the autonomous tertiary care hospital between January 2013 and August 2018. Records of all patients with CPSS who had been operated were reviewed.

Inclusion criteria

Children with extrahepatic CPSS Type 2 who had undergone shunt ligation, were included in the study. Their presentation and demographics were recorded. The presence of the shunt in these patients had been proven with Doppler ultrasonography of the abdomen; contrast-enhanced computed tomography (CECT) of the

Clinical presentation

Five patients were included in the study with a median age of 6 years (3.5 years to 13 years). Out of these five patients, four were boys, and one was a girl. The most common presentation was with hepatopulmonary syndrome (HPS) and was present in 4 patients (Table 1). All these four patients demonstrated central cyanosis, clubbing and a low SpO2 (60–70%) on room air. One of these patients had been previously reported [5]. One patient also had intermittent jaundice and anemia which required

Discussion

CPSS is a rare malformation that though initially described by Abernethy in 1793 [1], was further classified by Morgan and Superina in 1994 into Type 1 malformation, in which liver is not perfused with portal blood, and there is an end to side shunt. In Type 2 malformation the liver is still perfused due to partial shunting of blood through and side to side portosystemic shunt [6] (Fig. 4). The presentation and the age of presentation of this condition are very varied. CPSS has been diagnosed

Conclusion

Abernethy malformation is a very rare malformation. Patients may present with a hepatopulmonary syndrome, liver tumor, pulmonary artery hypertension, jaundice and rarely nephrotic syndrome. The shunt can be demonstrated easily on ultrasonography or computed tomography. Shunt occlusion angiography and liver biopsy may be needed to identify distal portal vein and its branches. Shunt ligation or occlusion either staged or in a single sitting is the treatment of choice. Postoperative elevation in

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Percutaneous sequential closure of an Abernethy malformation: A case report
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Abernethy malformation (congenital extrahepatic portosystemic shunt [CEPS]) is rare and is characterized by an aberrant connection between the portal and systemic veins, bypassing the liver. It can have varying presentations and can lead to severe complications if left untreated. It is usually diagnosed incidentally on abdominal imaging. Occlusion venography and measurement of portal pressures (pre- and postocclusion) is an important step in management. Complete occlusion of the malformation in cases where the portal veins in the liver are very small and the gradient is more than 10 mm Hg, can potentially lead to acute portal hypertensive complications, such as porto-mesenteric thrombosis. We report a case of Abernethy malformation diagnosed on an abdominal computed tomography scan that presented with neurological symptoms and was successfully managed by interventional radiology via endovascular closure through placement and sequential occlusion of 2 metal stents.
Diagnostic and monitoring value of ultrasound, computed tomography angiography, and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts
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To investigate the diagnostic and monitoring value of ultrasound (US), computed tomography angiography (CTA), and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts (CEPS) in children.
We retrospectively analysed different imaging examinations of 15 children with CEPS. Development of the portal vein before shunt occlusion, shunt location, portal vein pressure, main symptoms, diameter of the main portal vein, and location of secondary thrombosis after shunt occlusion were recorded. Final classification diagnosis was made by portal venography after shunt occlusion, and consistency with other imaging examinations in diagnosing development of the portal vein was calculated using Cohen’s kappa.
Compared with portal venography after shunt occlusion, US, CTA, and portal venography before shunt occlusion had poor consistency in showing the development of hepatic portal veins (Kappa value 0.091–0.194, P > 0.05). Six cases developed portal hypertension (40–48 cmH₂O) during the temporary occlusion test, and US showed that portal veins gradually expanded after shunt ligation. Eight patients with haematochezia had inferior mesenteric vein (IMV)-iliac vein (IV) shunts. After surgery, secondary IMV thrombosis was observed in eight cases and secondary splenic vein thrombosis in four cases.
Portal venography with occlusion testing is very important to accurately evaluate the development of the portal vein in CEPS. The portal vein needs to expand gradually, and partial shunt ligation surgery is necessary in cases diagnosed as portal vein absence or hypoplasia before occlusion testing to avoid severe portal hypertension. After shunt occlusion, US is effective in monitoring portal vein expansion, and both US and CTA can be used to monitor secondary thrombi. IMV-IV shunts can cause haematochezia and are prone to secondary thrombosis after occlusion.
An Unusual Cause of Left Lower Extremity Varicose Veins
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Abernethy Malformation: An Unusual Extrathoracic Cause of Chronic Hypoxemia in Pediatrics
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Citation Excerpt :
In addition, the latest research indicates that about half of the Abernethy malformation patients in whom histology of nodule lesions in the liver have been reported have focal nodular hyperplasia. However, it may develop into a neoplasm because of the absence of portal hepatotropic factors and the increased hepatic arterial flow secondary to portal ischemia.16,17 It is, therefore, necessary to evaluate these nodule lesions carefully.
Abernethy malformation is a very rare congenital vascular malformation consisting of diversion of portal blood away from liver, and it is commonly associated with multiple congenital anomalies. Here, we present a case of a male from China with nonspecific abdominal pain associated with an unusual pattern of type II Abernethy malformation, whose was diagnosed with a portosystemic shunt via a giant portal-inferior vena cava fistula (17.22 mm in diameter). The patient underwent a surgical ligation of the portocaval shunt and recovered well. We believe that this is the first case of a type II Abernethy malformation presenting as a portosystemic shunt via the giant portal-inferior vena cava fistula.
Successful surgical attenuation of portosystemic shunt in a dog with imaging-diagnosed portal vein aplasia
2024, Canadian Veterinary Journal

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^☆: Declarations of interest: None.

^☆☆: Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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Other ConditionAbernethy malformation type 2: varied presentation, management and outcome☆,☆☆

Abstract

Purpose

Material and methods

Results

Conclusion

Level of evidence

Section snippets

Study design

Inclusion criteria

Clinical presentation

Discussion

Conclusion

J Pediatr Surg

Hum Pathol

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Health Care

Account of two instances of uncommon formation, in the viscera of the human body

Philos Trans R Soc Lond

The Abernethy malformation-myriad imaging manifestations of a single entity

Indian J Radiol Imaging

Abernethy syndrome, a rare cause of hypoxemia: a case report

Ann Pediatr Cardiol

Complications of congenital portosystemic shunts in children: therapeutic options and outcomes

J Pediatr Gastroenterol Nutr

Polysplenia with pulmonary arteriovenous malformations

Pediatr Cardiol

Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report

Liver Transpl

Hepatic nodular lesions associated with abnormal development of the portal vein

AJR Am J Roentgenol

Successful surgical ligation under intraoperative portal vein pressure monitoring of a large portosystemic shunt presenting as an intrapulmonary shunt: report of a case

Surg Today

Other Condition
Abernethy malformation type 2: varied presentation, management and outcome☆,☆☆